Global Journal of Medical Research, A: Neurology & Nervous System, Volume 23 Issue 3

On June 2022, he had moderate dysarthria, hypomimia, right-hand flexor dystonia, tetraparesis, bradykinesia, and postural instability, but without rigid or resting tremors. The specific treatment was started on May 2022 with pyridoxine chlorhydrate 50 mg daily and zinc sulfate heptahydrate 4 mg/mL 15mL three times a day orally. Due to the cost of the drug, the patient delayed starting penicillamine. III. D iscussion Incipient neurological symptoms are subtle and nonspecific, such as difficulty concentrating and motor coordination and handwriting changes (for example, micrograph) 13-19 and begin on average between 20 and 40 years 20-22 . As it progresses, more prominent symptoms appear, whose order of incidence is dysarthria (57.6%), dystonia (42.4%), abnormal gait (37.8%), tremor (36.2%), parkinsonism (17.3%), choreoathetosis (15.3%) and convulsion (4.7%) 16,17 . Neurological impairment occurs about a decade after liver failure and, therefore, signs of advanced disease 23 . Cognitive impairment is considered rare and was reported by Machado, Chien, Deguti, et al. (2006) 16 in 4.2% of cases. Given the heterogeneity of clinical manifestations, the neurological phenotype of WD can be grouped for didactic purposes into dystonic, pseudosclerotic, parkinsonian, and hyperkinetic subtypes 4 . The patient discussed in this study had a predominance of the dystonic subtype manifested by multifocal dystonia affecting both the risorius muscle (sardonic laughter) and the flexor muscles of the right hand fingers. As reported by Lorincz (2010) 24 , bilateral putaminal lesions were found on an MRI of the brain. Dysarthria can result from any condition that damages the motor control structures necessary for speech production, such as cranial nerves IX, X, XII, cerebellum, and basal ganglia 12 . In this case, it was noted evident bilateral impairment of the basal ganglia. Seizures are not uncommon and are reported variably in 4.7% 16,17 to 14.5% 25 of WD cases. The patient in question presented, at the initial manifestation, a single episode of generalized tonic-clonic seizure without recurrence. We also detected the presence of brownish Kayser Fleischer rings, more evident in the lower region of the iris bilaterally. Such a semiological sign is due to copper deposition in the Descemet's membrane of the cornea 26 and is present in approximately 100% of neuropsychiatric WD cases 10 . Psychiatric symptoms are reported by about 30% to 60% of individuals affected by WD 26 . In this case, the disorder for which the patient had been using Fluoxetine was not specified. However, the familiar states that at the time of initiation of therapy, he had a depressive mood, anhedonia, and anxiety. It is possible that such symptoms were already an incipient manifestation of central nervous system involvement. Cognitive impairment is initially mild and recognized only by family members. It is categorized into frontal lobe syndrome, which involves impulsivity, promiscuity, apathy, hypotenacity, impaired social judgment, planning dysfunction, and emotional lability, and subcortical dementia characterized by slowed thinking amnesia, and executive dysfunction, but without aphasia, apraxia, or agnosia 10 . In this case, it was impossible to attribute a clinical syndrome related to the metabolic disorder, given the history of alcoholism and use of narcotics. IV. C onclusion This case report represents the importance of a detailed neurological clinical evaluation and the association of findings with Imaging and laboratory workup. It is a rare disease whose epidemiology in Brazil lacks data, and complementary tests have reduced specificity. 19 Year 2023 Global Journal of Medical Research Volume XXIII Issue III Version I ( D ) A © 2023 Global Journals Neurological Wilson Disease in a Young Brazilian Adult: A Case Report